Introduction
Hereditary spherocytosis (HS) is a hemolytic anemia of varying severity
caused by defects in the red blood cell membrane. Defects in the
erythrocyte membrane and selective destruction of defective erythrocytes
by the spleen are two key factors in the pathophysiological process of
HS. The destruction of the red blood cells by the spleen can lead to
proliferative enlargement of the spleen, which leads to increased red
blood cell accumulation and/or increased hemolysis, thus creating a
vicious cycle1.
The treatment of HS is aimed at minimizing the complications of chronic
hemolysis and anemia. In addition to symptomatic treatment and blood
transfusion, current guidelines recommend splenectomy if the patient has
severe anemia complications or is transfusion
dependent1–3. Since the spleen is a lymphoid organ,
the overwhelming post-splenectomy infection (OPSI) is the most serious
postoperative complication, especially in children. In addition to
surgical risks, splenic resection is also prone to serious surgical
complications such as venous thromboembolism (VTE), pulmonary arthritis,
and pulmonary artery hypertension (PAH) after
splenectomy4. Patients who have indications for
splenectomy but were unwilling to undergo surgery, or who have
contraindications to surgery, have been reported to be treated with PSE,
which exhibited better outcomes and fewer surgical complications due to
residual partial splenic immunity5,6. However, PSE had
not been widely studied and applied in pediatric HS patients due to the
poor controllability of the embolic range.
The aim of our study was to investigate another surgical procedure,
which can achieve an accurate prediction of the extent of splenic
embolism. We know that the splenic artery near the splenic hilum is
usually divided into two branches at the upper and lower ends, each of
which is further divided into 4-6 segmental intrapleural branches, with
few traffic branches between these different segments. Thus, we first
proposed SPSE to treat HS patients based on the anatomical
characteristics of the splenic artery. The present study reports on the
feasibility and efficacy of SPSE in a cohort of 16 children with HS.